Light and electron microscopy, histochemical study of acetylcholinesterase (ACE) were used in examination of the state and innervation of ocular muscles in autopsy material from a patient who died of amyotrophic sclerosis (ATS). The patient had lived 14 years under artificial lung ventilation, ATS was diagnosed 22 years before the death. Light microscopy demonstrated the intactness of the muscle fibers and the presence of three types characteristic of the extraocular muscles: thin, granular and rough. Besides typical differences structural changes were observed in some muscle fibers of the myopathic character and inclusions not limited by membrane having filiform or granular structure. Ocular muscles had an intensive innervation. Nervous fiber terminals revealed by a reaction for ACE were represented by single motor plaques and multiple cluster-like endings. Ultrastructurally, nervous endings of two types differed by terminals and fold expression on the postsynaptic membrane. There were no pathological changes in axons and myelin. Thus, ocular muscles were not affected as well as their nervous apparatus at completion of AMS, this indicating the noninvolvement of this muscular allotype in a specific degenerative process.